Chondroid Hamartoma Research Articles

Multiple Pulmonary Chondroid Hamartoma

A 30-year-old male was admitted to our hospital for a 3-month history of cough. The computed tomography (CT) scan of chest revealed multiple lesions with sporadic calcification (popcorn calcification) in the upper and middle lobes of the right lung (Fig. ​(Fig.1).1). A presumptive diagnosis of chondroid hamartoma was made according to the CT-guided percutaneous biopsy. Systemic positron emission tomography-CT (PET-CT) scan was also negative for other lesions. A posterolateral thoracotomy was performed and innumerous firm round yellowish-white nodules were found and spread over the right upper and middle lobes (Fig. ​(Fig.2,2, arrow). The two lobes involved were successfully excised. The final specimen was 25 cm × 15 cm × 10 cm in size, firm, and full of nodules (Fig. ​(Fig.3).3). Postoperative pathological examination confirmed the diagnosis of pulmonary chondroid hamartomas (Fig. ​(Fig.4).4). The patient was discharged 7 days after the surgery without any main complication, and there is no sign of recurrence 3 months later. FIGURE 1. Computed tomography scan of chest showed multiple lesions of the right upper lobe, whereas the lower lobe seemed to be tumor free. Popcorn calcification, a typical imaging manifestation of pulmonary chondroid harmatoma, could be observed. FIGURE 2. The two lobes involved (arrow) seemed to out of physical function because they had fused and presented abnormal color. FIGURE 3. More yellow-white granules were found after the dissecting of the specimen. FIGURE 4. The tumor was mainly composed of chondroid tissue which was surrounded by epithelium, adipose tissue, and smooth muscle tissue could also be observed (hematoxylin and eosin stain, 200X). Pulmonary chondroid hamartoma, as a subtype of pulmonary harmatoma, is defined when chondroid tissue predominates in the composition of the tumor. Harmatoma is the most common benign tumor of the lung, which accounts for 8% of pulmonary neoplasms. It is well known that harmatomas are composed of tissue elements normally found at that site, but which are growing in a disorganized mass, usually seem as single, round nodules with distinct boundaries.1 A cytogenetic analysis of the pulmonary hamartomas showed an abnormal karyotype and revealed recombinations between chromosomal bands 6p21 and 14q24,2 which supported that a hamartoma of the lung was a true neoplasm. Solitary pulmonary hamartoma is a common benign tumor that is usually seen in males, whereas multiple pulmonary hamartomas are rare and predominate in females.3,4 Only a few reports of multiple pulmonary chondroid hamartomas were described so far. Here we presented an extremely rare case of multiple pulmonary chondroid hamartomas with innumerous nodules in the right upper and middle lobes. Most patients with pulmonary hamartoma are asymptomic, whereas some patients may have respiratory symptoms such as hemoptysis, cough, phlegm, or chest pain.5 Generally speaking, a non-anatomical resection is curative for a benign lesion although the location of the lesion plays an important role in the decision of surgical type.1 Video-assisted thoracic surgery now seems to be a trend in dealing with benign tumors because of less morbidity of surgical complications and hospital stay compared with traditional thoracotomy. On the other hand, endobronchial hamartomas can be well managed by endoscopy and laser ablation.6

Fine needle aspiration cytology of giant chondroid hamartoma of lung with review of literature

Giant pulmonary hamartoma (diameter > 9cms) is a very uncommon benign tumor with only eleven reported cases. Here we report such a rare case of giant pulmonary hamartoma measuring 12cms in diameter occupying almost whole of the left hemithorax. CT guided FNAC was done in suspicion of a malignant tumor but the cytomorphological features were of a benign cartilaginous neoplasm. The cytological findings were correlated with the radiological features and a diagnosis of chondroid hamartoma was suggested on FNAC. The diagnosis was later confirmed by histopathological examination.

Giant pulmonary hamartoma: A rare entity

Hamartoma is one of the common benign tumor of lung. These lesions represent an abnormal proliferation and mixing of the normal components of the lung tissue. Most hamartomas are asymptomatic. We report a rare case of giant pulmonary hamartoma (12 cm in diameter) occupied almost whole of left hemithorax. Computed tomography guided fine-needle aspiration cytology revealed a benign cartilaginous neoplasm, suggestive of chondroid hamartoma. Tru-cut biopsy of the tumor confirmed the diagnosis. Complete resection of the tumor was performed. Patient was on a regular follow-up postoperatively for 6 months without any clinical and radiological relapse.

Pediatric Tracheal and Endobronchial Tumors<subtitle>An Institutional Experience</subtitle>

To report the pathologic findings in cases involving endotracheal and endobronchial tumors in the pediatric population and to describe the presenting symptoms and treatment modalities for endotracheal and endobronchial tumors. Retrospective chart review. Tertiary care children's hospital. The study included 14 patients with endotracheal and endobronchial tumors. Patients were selected if bronchoscopy was performed to obtain biopsy specimens from the trachea or bronchus. There were 14 cases that met the inclusion criteria between 1993 and 2009. The patients ranged in age from 4 to 18 years. The most common presenting symptom was recurrent pneumonia (n = 6), followed by wheezing or asthma that was unresponsive to treatment (n = 4). Nine lesions (64%) were malignant and 5 (36%) were benign. Of the malignant tumors, 5 (55%) were carcinoid, 3 (33%) were mucoepidermoid carcinoma, and 1 was adenoid cystic carcinoma. There were 1 or 2 cases of each of the following benign to intermediate malignant potential lesions: histoplasmosis nodules, chondroid hamartoma, pulmonary chondroma, and inflammatory myofibroblastic tumor. In 12 cases, definitive treatment included surgical resection. Three of these cases required postoperative chemotherapy and radiotherapy. The results of this series suggest that in the pediatric population tracheal and endobronchial tumors are most likely to be carcinoid tumors or mucoepidermoid carcinomas, both malignant processes. For patients with recurrent pneumonias or chronic wheezing, an occult tumor is a diagnostic consideration that may require additional studies.

Video-assisted thoracoscopic surgery lobectomy – early experience

Introduction: Video-assisted thoracoscopic surgery (VATS) lobectomy is the anatomical resection of a whole lobe of the lung followed by removal of the lymph nodes from the mediastinum using a thoracoscope and an access incision (small thoracotomy ≤ 5 cm) without using the rib spreader. Aim: To present the early experience with VATS lobectomy. Material and methods: Five patients were treated surgically using the VATS technique of anatomical lung resection at the 2 nd Department of Surgery, Pavol Jozef Safarik University, University Hospital of L. Pasteur, Kosice, (Slovak Republic) within 12 months from 10.2008 to 10.2009. Lobectomy was performed in 4 patients and pneumonectomy in 1 patient. Results: The mean operating time was 120 min (range 80-170 min). Following lobectomy a drain was inserted into the pleural cavity in 4 cases, whereas there was no drainage after 1 pneumonectomy. Drains were removed 2-5 days after the surgery. Four patients suffered from lung carcinoma (1 squamous cell carcinoma, 3 adenocarcinomas); 1 patient had chondroid hamartoma. Postoperative condition was good in all patients. There were no early complications and patients were released home on the 6 th postoperative day on average. Conclusions: The advantages of VATS lobectomy have been widely discussed. There is a consensus that in elderly patients with non-small cell lung cancer VATS lobectomy accompanied by mediastinal lymphadenectomy reduces the incidence of complications after the surgery and patients recover faster.

Diffuse chondroid malformation of the lung: Cases series of a hitherto undescribed congenital lung disease

Congenital chondroid lesions of the lung are rare pathological findings. They are a constant feature of lung malformations such as giant cystic pulmonary chondroid hamartoma, chondroid cystic malformation, and in the "cartilaginous variant" of congenital adenomatoid malformation. All of these present as a large single thoracic mass.We present the cases of three males and two females with hitherto undescribed diffuse chondroid lung disease, all but one of whom had neonatal respiratory distress syndrome with interstitial syndrome on chest radiograph. The pathological findings were similar in all patients, showing large areas of disorganized lung parenchyma containing diffusely distributed mature cartilage islands. With a mean follow-up of 6 years, all patients had a favorable outcome. This diffuse chondroid lung disease appears to be a new entity whose initial presentation mimicked interstitial lung disease without the usual clinical, radiological, and histological features. We speculate that it could be part of a clinical spectrum between malformative chondroid lung cyst and congenital pulmonary airway malformation.

Computed Tomography Guided Percutaneous Thorasic: Fine Needle Aspiration Cytology in Lung and Mediastinum

A prospective and retrospective hospital based descriptive study was done to know the pathological spectrum of thoracic lesions and to correlate the radiological findings with cytological findings obtained from computed tomography guided percutaneous transThoracic fine needle aspiration of lung and mediastinum. The clinical, radiological and cytological data of 60 patients were studied who underwent CT guided FNAC from January 2008- June 2009. Diagnostic accuracy of FNAC is more than 90%. Out of 60 cases 19 cases are non malignant and 41 cases were malignant. The Diagnostic sensitivity of Bronchogenic carcinoma is 84% and Specificity is 76%. Male to Female ratio is 2.3:1. Majority of cases were seen in 5th and 6th decade. Apart from Non small cell carcinoma cases like small cell carcinoma, Carcinoid tumor, Chondroid hamartoma, SFT were also noted. Cytological diagnosis is correlated with cell block sections in available cases. Post procedural complications like Pneumothorax, Pulmonary hemorrhage and hemoptysis were noted in few cases. CT guided FNAC is a simple and safe procedure with high diagnostic accuracy in the evaluation of chest lesions. Pneumothorax, hemorrhage and hemoptysis are usually encountered complication. Very few cases require active management.

A Lung Mass Causing Cardiovascular Impairment

A Lung Mass Causing Cardiovascular Impairment

Congenital Nasal Chondromesenchymal Hamartoma

Objectives: To report the case of a congenital nasal chondromesenchymal hamartoma in a one-year-old female and review the literature, identifying problems encountered in confirming the diagnosis and in treatment of this patient.
 Methods:
 Design: Case Report 
 Setting: Tertiary Public General Hospital
 Patient: One
 Results: A one-year-old female with an intranasal mass noted at birth and with subsequent unilateral maxillary enlargement is described. Computed tomography showed calcifications and erosion of adjacent bony structures. Histopathology and immunohistochemistry of an intranasal biopsy were interpreted as chordoma, a malignant tumor. Following surgical excision, the final histopathologic diagnosis was chondroid hamartoma.
 Conclusion: Only 20 cases of nasal chondromesenchymal hamartoma have been reported in the literature worldwide. These tumors may present clinically, histopathologicaly and radiologically as malignant tumors and may mislead even the experts. The whole clinical picture should be taken together to avoid misdiagnosis as a malignancy and to facilitate appropriate management. 
 Keywords: nasal chondromesenchymal hamartoma, nasal masses in infancy, nasal chondroid lesions

Endobronchial chondroid hamartoma in an infant

Endobronchial tumors in infants are uncommon. The clinical and radiologic findings and management of a rare case of endobronchial chondroid hamartoma in an infant is presented along with a review of the literature.

“Popcorn” Calcifications in a Pulmonary Chondroid Hamartoma

A 22-year-old man was referred for evaluation of an asymptomatic mass in the left upper lung that was detected on a screening chest radiograph.

Abundant cartilage formation of congenital pulmonary airway malformation – A case report

Congenital pulmonary airway malformation (CPAM) of the lung is an uncommon developmental anomaly. We report an unusual case of type 1 CPAM with abundant cartilage in a 5-year-old boy. On chest radiography, a left lung mass was detected incidentally, and tumor resection was performed under the impression of a benign tumor. The pathological examination of the mass revealed abundant cartilage in the walls of malformed bronchioles with partially cystic dilatation. We think that this case represents a cartilaginous variant of CPAM. The cartilaginous variant of CPAM should be differentiated pathologically from other pulmonary neoplasms containing abundant cartilage, such as chondroid hamartoma.

Giant cystic chondroid hamartoma of the lung

Pulmonary hamartoma composed of an abnormal mixture of mesenchymal elements is the most common benign neoplasm in the lung. Pulmonary hamartoma larger than 10 cm and the cystic variant are also very rare. We describe an asymptomatic 11-year-old boy with a huge mass in the right hemithorax. Chest computed tomography revealed a heterogeneous mass filling the middle and the lower lobes of the right lung. The patient underwent thoracotomy and resection of the lesion. Gross examination of the surgical specimen showed a well-circumscribed, encapsulated, lobulated, and bluish-white mass, measuring 18 × 16 × 8 cm. Multilocular cystic spaces with intervening lobulated fragments of cartilaginous tissue and adipose tissue were seen. Microscopically, the solid component was composed of cartilage and adipose tissue. Cystic areas and cleft-like spaces were lined by ciliated columnar epithelium. We believe this is the first case of ‘giant cystic chondroid hamartoma of the lung’ described in childhood. We suggest that giant cystic pulmonary hamartoma should be included in the differential diagnosis of large intrathoracic masses in children.

Giant chondroid hamartoma in a 19‐year‐old girl

Giant chondroid hamartoma in a 19‐year‐old girl

Omental lipoblastoma

A large intra-abdominal mass was discovered in a 6-month-old boy during a routine well-child examination. Imaging studies revealed a solid mass which appeared to arise from the left lobe of the liver, extending caudally and filling the entire pelvis. At the time of surgical excision, the mass was found to be unassociated with the liver, but was instead localized to the omentum. Histologic examination revealed adipocytes of varying stages of maturation arranged in a lobular architecture, consistent with a lipoblastoma. This unusual tumor is only the eighth reported omental lipoblastoma [J. Hicks, A. Dilley, D. Patel, J. Barrish, S. Zhu, M. Brandt, Lipoblastoma and lipoblastomatosis in infancy and childhood: histologic, ultrastructural, and cytogenetic features. Ultrastruct. Pathol. 25 (2001) 321–333; J. Harrer, G. Hammon, T. Wagner, M. Bolkenius, Lipoblastoma and lipoblastomatosis: a report of two cases and review of the literature. Eur. J. Pediatr. Surg. 11 (2001) 342–349; S. Weiss and J. Goldblum, Enzinger and Weiss's Soft Tissue Tumors, fourth ed., Mosby, St. Louis, MO, 2001, pp. 601–605, 670–686; S. Soin, S. Andronikou, R. Lisle, K. Platt, K. Lakhoo, Omental lipoblastoma in a child; diagnosis based in CT density measurements. J. Pediatr. Hematol. Oncol. 28(1) (2006) 57–58; A. Prando, S. Wallace, J.L. Marins, R.M. Pereira, E.R. de Oliveira, M. Alvarenga, Sonographic features of benign intraperitoneal lipomatous tumors in children—report of 4 cases. Pediatr. Radiol. 20(8) (1990) 571–574; C. Blank, E. Schoenmakers, P. Rogalla, E. Huys, A. Van Rijk, N. Drieschner, J. Bullerdiek, Intragenic breakpoint within RAD51L1 in a t(6;14)(p21.3;q24) of a pulmonary chondroid hamartoma. Cytogenet. Cell Genet. 95 (2001) 17–19; S. Ingraham, R. Lynch, S. Kathiresan, A. Buckler, A. Menon, hREC2, a RAD51-like gene, is disrupted by t(12;14)(q15;q24.1) in a uterine leiomyoma. Cancer Genet. Cytogenet. 115 (1999) 56–61]. Cytogenetics revealed a karyotype of 46, XY, t(8;14)(q13;q24). While lipoblastomas characteristically involve 8q, only one prior case has been reported with 14q24 as its fusion partner [M. He, K. Das, M. Blacksin, J. Benevenia, M. Hameed, A translocation involving the placental growth factor gene is identified in an epithelioid hemangioendothelioma. Cancer Genet. Cytogenet. 168 (2006) 150–154]. We report this unique case of an omental lipoblastoma with a focus on its unusual karyotype, as well as its differentiation from myxoid liposarcoma.

Massive chondroid hamartoma of the lung clinically masquerading as bronchogenic carcinoma

Chondroid hamartomas of the lung are uncommon lesions which are generally small sized and asymptomatic. Herein we describe a case of a large-sized pulmonary chondroid hamartoma which clinically mimicked bronchogenic carcinoma. A large hilar growth was detected in the left lung on radiological studies. Left upper lobectomy was done. The growth was well defined with a lobulated bluish cut surface. Histopathology disclosed lobules of mature cartilage rimmed by long slit-like epithelial channels admixed with mature adipose tissue.

Radiofrequency Thermoablation in Chest Wall Mesenchymal Hamartoma of an Infant

We report on an infant presenting with a chondroid hamartoma managed with a combined conservative surgical treatment and radiofrequency thermoablation.

Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

To highlight the variation in clinical manifestations, imaging and management of four cases of unilateral pulmonary artery agenesis presenting in adulthood. Four patients with unilateral pulmonary artery agenesis were referred to our institution between 1995 and 2005. They underwent a series of investigations, including chest radiography, echocardiography, ventilation perfusion scintigraphy, angiography, computed tomography (CT) and magnetic resonance imaging (MRI). Two of the four patients had absence of the right main pulmonary artery, whilst the remaining two patients had absence of the left main pulmonary artery. One patient showed a restrictive defect on pulmonary function tests. Two patients who had ventilation perfusion scintigraphy showed absent perfusion and reduced ventilation on the affected side. Angiography (where performed), CT and MRI confirmed the anatomy and the presence of multiple collaterals. Bronchiectasis was demonstrated on CT in two patients, with one also demonstrating a mosaic attenuation pattern. One patient had an incidental lung tumour on the side of the agenesis, which was diagnosed as a chondroid hamartoma on histology. Three of the four patients eventually underwent resection of the affected lung. Isolated unilateral pulmonary artery agenesis has a non-specific presentation. Awareness of this condition can lead to earlier diagnosis, with cross-sectional imaging making an important contribution.

Contrast-enhanced ultrasound (CEUS) for the study of peripheral lung lesions: A preliminary study

The use of contrast-enhanced ultrasound (CEUS) for the study of peripheral lung lesions has never been systematically investigated. We evaluated the CEUS patterns of 98 peripheral lung lesions before performing US-guided fine-needle biopsies (FNB). The examinations were done with an Esaote Technos MPX scanner in the harmonic mode with a mechanical index of 0.04 or less. Contrast enhancement was achieved with a 4.8 mL bolus of SonoVue (Bracco) administered via an antecubital vein. All FNBs performed under CEUS guidance were adequate for pathologic diagnosis. Seventy-eight lesions were malignant: 33 (41.9%) were adenocarcinomas, 29 (36.5%) were squamous-cell carcinomas, 13 (17.6%) were undifferentiated large-cell carcinomas and the remaining three (4.1%) were small-cell carcinomas. All presented intralesional enhancement consistent with tumor neovascularization. In some cases, there were unenhanced areas consistent with zones of necrosis and these areas were avoided during FNB. The other 20 lesions were benign (four lipomas, two fibrous lung tumor, two noncaseous granulomas, six abscesses, one rheumatoid nodule, one histiocytosis X, one chondroid hamartoma, one sclerosing hemangioma, two sarcoid nodules) and none presented intralesional enhancement. This initial and admittedly limited experience suggests that CEUS may provide diagnostically useful information on peripheral lung lesions and increase the diagnostic yield of transthoracic FNB by reducing the risk of inadequate tissue sampling. (E-mail: sperandeom@libero.it)

Fusion of the HMGA2 and NFIB genes in lipoma

The major cytogenetic subgroup of lipomas is characterized by aberrations of chromosome segment 12q13-15, which recombines with a large number of other chromosomal regions. The gene HMGA2 is the main target in these aberrations. For some recurrent rearrangements, chimeric transcripts, including the 5' part of HMGA2, have been described. The 3' partners identified are LPP, LHFP, CMKOR1, and EBF. In addition, subsets of other benign solid tumors show aberrations of 12q13-15. Among pleomorphic adenomas of the salivary glands, where the preferred recombination partner with 12q13-15 is 9p22-24, an HMGA2/NFIB fusion gene has been reported. In the present study, two cases of lipoma with rearrangements of 9p22-24 and 12q15 were analyzed by reverse transcription polymerase chain reaction to find out if HMGA2/NFIB was also present in lipoma. An in-frame fusion transcript, combining the four first exons of HMGA2 with exon 8 of NFIB, was detected in one case. It was identical to a transcript that was previously described in salivary gland adenoma and contained a stop codon shortly 3' of the fusion point. The finding of the same fusion gene in different tumors is not unique. For example, HMGA2/LPP has been reported in lipoma, pulmonary chondroid hamartoma, and soft tissue chondroma. Since similar 9;12 translocations have been described also in rare cases of hamartoma and uterine leiomyoma, the occurrence of HMGA2/NFIB could be postulated in these tumors as well.